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Aortic valve replacement options

Hello,

I wrote a little while back about my daughter age three who was diagnosed at birth with an ASD, VSD, and W.P.W. syndrome. The W.P.W. was ablated in January '09. Her VSD was closed with open heart surgery in May 2009, because there was concern it was damaging her aortic valve (classified with mild regurgitation at the time). Since her VSD surgery, her valve has deteriorated enough that she is looking at surgery for her valve in the next few months. We have a meeting next week to discuss it further. My understanding is there are three main options:

Ross Procedure
Tissue Valve
Mechanical Valve

I know you cannot comment on my daughter's specific case, but I am wondering if you can shed some light the general parameters in which one option becomes the recommended choice or specifically contraindicated? I know they all have their pros and cons, so I am trying to understand what makes one option a better fit for a patient over the others? At this point we are very opposed to the idea of a mechanical valve given her age, her gender, and her activity level. What should make us reconsider it? We also understand that the Ross procedure requires the most expertise, but how do we know what constitutes "experience" doing the Ross Procedure? A surgeon who has done 20? 200? How do we go about determining whether or not a hospital/surgeon would be capable of doing the Ross? What makes a tissue valve a good option?

Dear rjthkids: It is a difficult situation for a pediatric cardiologist to recommend open heart surgery for asymptomatic patients, as in your daughter’s case. There is always some risk to open heart surgery. The balance is in determining if the risk of open heart surgery outweighs the risk of leaving the patient with a cardiac condition that has either progressed, or is associated with known long term risks of heart or valve dysfunction. In your daughter’s case, the prolapse of the aortic valve cusp into the VSD is a known complication of spontaneous attempts by the body to close a VSD naturally. The development of aortic valve leakage is a further known complication of that situation. Unfortunately, in some cases, the actual VSD surgical repair can further distort the aortic valve, and in some cases, a valve leaks more after the surgery than it did before. The decision to re-operate on the valve depends upon how severe the valve leakage is, whether or not there is enlargement of the left ventricle, and whether there are associated clinical symptoms. Your daughter is quite young and may be able to tolerate some degree of valve leakage for a while before needing another operation, but that must be decided by your cardiologist.
With regards to options for aortic valve surgery, in all likelihood, your daughter is too small for even the smallest mechanical (artificial) valve. That then leaves 3 options: 1) an aortic valvuloplasty where the surgeon does a “plastic surgery” on her valve to try to tighten it up so it does not leak as much, 2) a Ross procedure where her own pulmonary valve may qualify to replace her aortic valve, but a homograft/tissue valve needs to be placed in the position of the of pulmonary valve, and 3) replacement of her aortic valve with a homograft or tissue valve. Option 1 (valvuloplasty) cannot always be guaranteed to be successful because it depends upon the characteristics of the valve. Option 2 (Ross) is used often in small children, because the “neo-aortic valve” can grow with the patient. But the valve can also develop severe leakage in 5-10% of patients and need another operation. The lifespan of the valve could be 15 years or more. However, the homograft valve in the pulmonary position has its own set of problems (blockage, leakage) that may require surgery there. So the Ross procedure is not perfect either. Option 3 (homograft or tissue valve replacement) is often done in children of young ages because these valves come in many sizes, and do not require the use of anticoagulation; but the preserved valve will deteriorate and need replacement after about 10-15 years (sometimes sooner). Therefore, please make sure you discuss all of these things thoroughly with your pediatric cardiologist and your child’s heart surgeon. Valve surgery during childhood is not as straight forward as it is in grown adults, since the child is still subject to further growth. Often this is not an emergency surgery, and you should think about getting a second opinion from a large pediatric cardiology center that does several hundred open heart cases annually.

I also have a slightly separate question. When my daughter was born, her VSD was classified as moderate in size at 6mm, located right next to her valve. Through the next 2 1/2 years it was believed to be actively closing and towards the end to be 3mm in size, functioning at 1 mm in size due to tissue. She showed absolutely no symptoms and her aortic regurgitation was stable the entire time at a mild level. When we went ahead and did the surgery to close the valve because her cardiologist felt the murmur was getting louder, it turned out her VSD was actually 10 mm in size and had been plugged by her aortic valve that entire time.

She had 2 1/2 asymptomatic years with a stable valve and a 10 mm VSD; we closed her VSD and now within six months her valve has degraded enough to need surgery. I'm confused as to why the surgery we did for to protect her valve seems to have made everything dramatically worse, instead of better? Why would a valve do so well with an open 10 mm VSD, and worse after corrective surgery, generally speaking?

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